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Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure
Dhruti P Chen1, Margaret E Helmuth2, Abigail R Smith2
1University of North Carolina, Chapel Hill, and Duke Children's Hospital Medical Center, Durham, North Carolina.
Rationale & Objective:
Adolescent- and adult-onset minimal change disease (MCD) may have a clinical course distinct from childhood-onset disease. We characterized the course of children and adults with MCD in the Cure Glomerulonephropathy Network (CureGN) and assessed predictors of rituximab response.
Study Design:
Prospective, multicenter, observational study.
Study Participants:
CureGN participants with proven MCD on biopsy.
Exposure:
Age at disease onset, initiation of renin-angiotensin-aldosterone system (RAAS) blockade, and immunosuppression including rituximab during the study period.
Outcome:
Relapse and remission, change in estimated glomerular filtration rate (eGFR), and kidney failure.
Analytical Approach:
Remission and relapse probabilities were estimated using Kaplan-Meier curves and gap time recurrent event models. Linear regression models were used for the outcome of change in eGFR. Cox proportional hazards models were used to estimate the association between rituximab administration and remission.
Results:
The study included 304 childhood- (≤12 years old), 49 adolescent- (13-17 years old), and 201 adult- (≥18 years) onset participants with 2.7-3.2 years of follow-up after enrollment. Children had a longer time to biopsy (238 vs 23 and 36 days in adolescent- and adult-onset participants, respectively; P<0.001) and were more likely to have received therapy before biopsy. Children were more likely to be treated with immunosuppression but not RAAS blockade. The rate of relapse was higher in childhood- versus adult-onset participants (HR, 1.69 [95% CI, 1.29-2.21]). The probability of remission was also higher in childhood-onset disease (HR, 1.33 [95%CI, 1.02-1.72]). In all groups eGFR loss was minimal. Children were more likely to remit after rituximab than those with adolescent- or adult-onset disease (adjusted HR, 2.1; P=0.003). Across all groups, glucocorticoid sensitivity was associated with a greater likelihood of achieving complete remission after rituximab (adjusted HR, 2.62; P=0.002).
Limitations:
CureGN was limited to biopsy-proven disease. Comparisons of childhood to nonchildhood cases of MCD may be subject to selection bias, given that childhood cases who undergo a biopsy may be limited to patients who are least responsive to initial therapy.
Conclusions:
Among patients with MCD who underwent kidney biopsy, there were differences in the course (relapse and remission) of childhood-onset compared with adolescent- and adult-onset disease, as well as rituximab response.
Plain-Language Summary:
Minimal change disease is a biopsy diagnosis for nephrotic syndrome. It is diagnosed in childhood, adolescence, or adulthood. Patients and clinicians often have questions about what to expect in the disease course and how to plan therapies. We analyzed a group of patients followed longitudinally as part of the Cure Glomerulonephropathy Network (CureGN) and describe the differences in disease (relapse and remission) based on the age of onset. We also analyzed rituximab response. We found that those with childhood-onset disease had a higher rate of relapse but also have a higher probability of reaching remission when compared with adolescent- or adult-onset disease. Children and all steroid-responsive patients are more likely to achieve remission after rituximab.
Insights
Children with minimal change disease (MCD) experience more relapses but have a higher remission probability than adolescents and adults. Steroid-responsive patients and children show better outcomes with rituximab treatment for MCD.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Immunology
Background:
- Minimal change disease (MCD) is a leading cause of nephrotic syndrome in children and can also affect adolescents and adults.
- The clinical course and treatment response of MCD may vary significantly based on the age of disease onset.
- Understanding these differences is crucial for optimizing patient management and therapeutic strategies.
Purpose of the Study:
- To characterize the clinical course of minimal change disease (MCD) in relation to age at onset.
- To compare relapse rates, remission probabilities, and estimated glomerular filtration rate (eGFR) changes across childhood, adolescent, and adult-onset MCD.
- To assess predictors of rituximab response in MCD patients.
Main Methods:
- A prospective, multicenter, observational study was conducted within the Cure Glomerulonephropathy Network (CureGN).
- Participants with biopsy-proven MCD were analyzed based on age at onset: childhood (≤12 years), adolescent (13-17 years), and adult (≥18 years).
- Kaplan-Meier curves, recurrent event models, linear regression, and Cox proportional hazards models were used to analyze outcomes including relapse, remission, and eGFR changes, with a focus on rituximab response.
Main Results:
- The study included 304 childhood, 49 adolescent, and 201 adult-onset MCD cases with a median follow-up of 2.7-3.2 years.
- Childhood-onset MCD exhibited a higher relapse rate (HR, 1.69) and remission probability (HR, 1.33) compared to adult-onset disease.
- Children were more likely to achieve remission after rituximab (adjusted HR, 2.1), and glucocorticoid sensitivity predicted better rituximab response (adjusted HR, 2.62) across all age groups.
Conclusions:
- Age at onset significantly influences the clinical course of minimal change disease (MCD), with childhood-onset cases showing distinct patterns of relapse and remission.
- Rituximab demonstrates efficacy in achieving remission, particularly in children and steroid-sensitive patients.
- These findings highlight the importance of considering age at onset when managing MCD and tailoring immunosuppressive therapies.
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